Diagnosis of Primary Hypophysitis in Germany

Honegger, Juergen; Schlaffer, Sven; Menzel, Christa; Droste, Michael; Werner, Sandy; Elbelt, Ulf; Strasburger, Christian J.; Stoermann, Sylvère; Kueppers, Anna; Werf, Christine Streetz-van der; Deutschbein, Timo; Stieg, Mareike R.; Rotermund, Roman; Milian, Monika; Petersenn, Stephan

doi:10.1210/jc.2015-2152

Cited by 106 publications

(116 citation statements)

References 33 publications

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“…Infiltrative lesions, however, like lymphocytic and granulomatous hypophysitis are largely managed conservatively, except when symptoms are severe or progressive -in such cases surgery may be performed or corticosteroids may be used. (63,78,79,80) Primary lymphomas in the region are managed with chemotherapy and radiotherapy and rarely surgically, although stereotactic biopsy may be needed for accurate diagnosis (61).…”

Section: To Treat or Not To Treat?mentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Pituitary ‘incidentaloma’: neuroradiological assessment and differential diagnosis

Васильев

Rostomyan

Daly

et al. 2016

European Journal of Endocrinology

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Pituitary incidentalomas are a by-product of modern imaging technology. the term 'incidentaloma' is neither a distinct diagnosis nor a pathological entity. Rather, it is a collective designation for different entities that are discovered fortuitously, requiring a working diagnosis based on the input of the radiologist, endocrinologist and often a neurosurgeon. In addition to pathological conditions affecting the pituitary gland, a thorough knowledge of the radiological characteristics of normal variants and technical artifacts is required to arrive at an accurate differential diagnosis. after careful radiological and hormonal evaluation, the vast majority of pituitary incidentalomas turn out to be non-functioning pituitary microadenomas and Rathke's cleft cysts (Rccs). Based on the low growth potential of non-functioning pituitary microadenomas and Rccs, periodic MRI surveillance is currently considered the optimal management strategy. stricter follow-up is required for macroadenomas, as increases in size occur more frequently.

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Section: To Treat or Not To Treat?mentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Pituitary ‘incidentaloma’: neuroradiological assessment and differential diagnosis

Васильев

Rostomyan

Daly

et al. 2016

European Journal of Endocrinology

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“…Autoantibodies emerged during the first 18 months after the NE. The APAs were negative, but the diagnostic sensitivity and specificity of APA tests are known to be low (12, 13). …”

Section: Discussionmentioning

confidence: 99%

“…The typical order of trophic hormone deficiency has been reported as ACTH > TSH > FSH/LH > PRL > GH (12). However, the recent report with 76 cases from Germany found hypogonadotrophic hypogonadism, the most frequent hormonal defect in primary hypophysitis (13). Our patient first started to experience symptoms suggestive of hypogonadism, three months after NE.…”

Section: Discussionmentioning

confidence: 99%

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Autoimmune polyendocrinopathy and hypophysitis after Puumala hantavirus infection

Tarvainen

Mäkelä

Mustonen

et al. 2016

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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SummaryPuumala hantavirus (PUUV) infection causes nephropathia epidemica (NE), a relatively mild form of haemorrhagic fever with renal syndrome (HFRS). Hypophyseal haemorrhage and hypopituitarism have been described in case reports on patients with acute NE. Chronic hypopituitarism diagnosed months or years after the acute illness has also been reported, without any signs of a haemorrhagic aetiology. The mechanisms leading to the late-onset hormonal defects remain unknown. Here, we present a case of NE-associated autoimmune polyendocrinopathy and hypopituitarism presumably due to autoimmune hypophysitis. Thyroid peroxidase antibody seroconversion occurred between 6 and 12 months, and ovarian as well as glutamate decarboxylase antibodies were found 18 months after acute NE. Brain MRI revealed an atrophic adenohypophysis with a heterogeneous, low signal intensity compatible with a sequela of hypophysitis. The patient developed central (or mixed central and peripheral) hypothyroidism, hypogonadism and diabetes insipidus, all requiring hormonal replacement therapy. This case report suggests that late-onset hormonal defects after PUUV infection may develop by an autoimmune mechanism. This hypothesis needs to be confirmed by prospective studies with sufficient numbers of patients.Learning points:Pituitary haemorrhage resulting in hypopituitarism has been reported during acute HFRS caused by PUUV and other hantaviruses.Central and peripheral hormone deficiencies developing months or years after HFRS have also been found, with an incidence higher than that in the general population. The pathogenesis of these late-onset hormonal defects remains unknown.This case report suggests that the late-onset hypopituitarism and peripheral endocrine defects after HFRS could evolve via autoimmune mechanisms.The sensitivity of current anti-pituitary antibody (APA) tests is low. A characteristic clinical course, together with typical brain MRI and endocrine findings may be sufficient for a non-invasive diagnosis of autoimmune hypophysitis, despite negative APAs.

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“…Eine lymphozytäre Hypophysitis mit Parenchymuntergang ist das typische morphologische Korrelat einer autoimmunassoziierten Entzündung und wird gelegentlich in Operationsprä-paraten vermeintlicher Hypophysentumoren gefunden [9].…”

Section: Histologische Veränderungen Bei Pgasunclassified

Polyglanduläre autoimmune Syndrome

Komminoth

2016

Pathologe

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Polyglandular autoimmune syndromes (PGAS), also known as autoimmune polyendocrinopathy syndromes (APS), are a heterogeneous group of rare, genetically caused diseases of the immune system which lead to inflammatory damage of various endocrine glands resulting in malfunctions. In addition, autoimmune diseases of non-endocrine organs may also be found. Early diagnosis of PGAS is often overlooked because of heterogeneous symptoms and the progressive occurrence of the individual diseases. The two most important forms of PGAS are the juvenile and adult types. The juvenile type (PGAS type 1) is caused by mutations in the autoimmune regulator (AIRE) gene on chromosome 21, exhibits geographic variations in incidence and is defined by the combination of mucocutaneous candidiasis, Addison's disease and hypoparathyroidism. In addition, autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) syndrome and other autoimmune diseases can also occur. The adult form of PGAS (PGAS type 2) is a multigenetic disorder associated with some HLA haplotypes, is more common than the juvenile type, shows female predominance and exhibits the combination of type 1 diabetes, autoimmune thyroid disease, Addison's disease and other autoimmune disorders. The histological alterations in affected organs of PGAS patients are similar to findings in sporadically occurring autoimmune diseases of these organs but there are no pathognomic fine tissue findings. If patients exhibit autoimmune changes in two different endocrine glands or if there are indications of several autoimmune disorders from the patient history, it is important to consider PGAS and inform the clinicians of this suspicion.

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Diagnosis of Primary Hypophysitis in Germany

Cited by 106 publications

References 33 publications

MANAGEMENT OF ENDOCRINE DISEASE: Pituitary ‘incidentaloma’: neuroradiological assessment and differential diagnosis

MANAGEMENT OF ENDOCRINE DISEASE: Pituitary ‘incidentaloma’: neuroradiological assessment and differential diagnosis

Autoimmune polyendocrinopathy and hypophysitis after Puumala hantavirus infection

Polyglanduläre autoimmune Syndrome

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